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Rabbit Anti-RAB3GAP2/PE Conjugated antibody (bs-11939R-PE)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-11939R-PE
英文名稱1 Rabbit Anti-RAB3GAP2/PE Conjugated antibody
中文名稱 PE標記的RAB3-GTP酶激活蛋白催化亞單位2抗體
別    名 DKFZp434D245; FLJ14579; KIAA0839; p150; Rab3 GAP p150; Rab3 GAP regulatory subunit; Rab3 GAP150; Rab3 GTPase activating protein 150 kDa subunit; Rab3 GTPase activating protein non catalytic subunit; RAB3 GTPase activating protein subunit 2 (non catalytic); RAB3 GTPase activating protein subunit 2; RAB3GAP150; RGAP iso; RP11 568G11.1; RBGPR_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  發(fā)育生物學  神經(jīng)生物學  信號轉導  G蛋白信號  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 156kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human RAB3GAP2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
ab 3 GAP p150 is a ubiquitously expressed protein that contains 1,393 amino acids and belongs to the Rab3-GAP regulatory subunit family. Defects in Rab 3 GAP p150 are the cause of Martsolf and Warburg Micro syndrome. Both syndromes are characterized by congenital cataracts, microphthalmia, postnatal microcephaly and developmental delay, and are inherited in an autosomal recessive manner. The Rab3 GTPase-activating complex is a heterodimer composed of RAB3GAP and Rab 3 GAP p150 that interacts with DMXL2. Existing as two alternatively spliced isoforms, the Rab 3 GAP p150 gene is conserved in chimpanzee, dog, cow, rat, chicken, zebrafish, fruit fly, mosquito, A.thaliana and rice. The Rab 3 GAP p150 gene contains 36 exons and maps to human chromosome 1q41.

Function:
RAB3GAP2 is a regulatory subunit of a GTPase activating protein that has specificity for Rab3 subfamily (RAB3A, RAB3B, RAB3C and RAB3D). Rab3 proteins are involved in regulated exocytosis of neurotransmitters and hormones. Rab3 GTPase-activating complex specifically converts active Rab3-GTP to the inactive form Rab3-GDP. It is required for normal eye and brain development and may participate in neurodevelopmental processes such as proliferation, migration and differentiation before synapse formation, and non-synaptic vesicular release of neurotransmitters.

Subunit:
The Rab3 GTPase-activating complex is a heterodimer composed of RAB3GAP and RAB3-GAP150. The Rab3 GTPase-activating complex interacts with DMXL2

Subcellular Location:
Cytoplasm. Note=In neurons, it is enriched in the synaptic soluble fraction.

Tissue Specificity:
Ubiquitous.

DISEASE:
efects in RAB3GAP2 are the cause of Martsolf syndrome (MARTS) [MIM:212720]. Martsolf syndrome is characterized by congenital cataracts, mental retardation, and hypogonadism. Inheritance is autosomal recessive.
Defects in RAB3GAP2 are the cause of Warburg micro syndrome type 2 (WARBM2) [MIM:614225]. WARBM2 is a rare syndrome characterized by microcephaly, microphthalmia, microcornia, congenital cataracts, optic atrophy, cortical dysplasia, in particular corpus callosum hypoplasia, severe mental retardation, spastic diplegia, and hypogonadism.

Similarity:
Belongs to the Rab3-GAP regulatory subunit family.

Database links:

Entrez Gene: 25782 Human

Entrez Gene: 98732 Mouse

Entrez Gene: 289350 Rat

Omim: 609275 Human

SwissProt: Q9H2M9 Human

SwissProt: Q8BMG7 Mouse

SwissProt: Q5U1Z0 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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